Behcet’s disease (Silk Road disease): a rare inflammatory disease

Background: Behcet’s disease (Silk Road disease), a rare immune-mediated multisystem inflammatory disorder described by intermittent oralaphthae and genital ulcer, backsliding uveitis, mucocutaneous, articular, gastrointestinal, neurological and vascular manifestations, with no cure.It is brought about by changes in the: a) arteries that flexibly blood to the body tissues b) veins that return the blood to the lungs, the rear of the eyes retina, brain, joints, skin and bowels. Case Presentation: A 55-year-old male patient was sensed with c/o joint agony in lower appendages, oral ulcer and scrotal ulcer. On physical assessment the patient was cognizant and oriented with B/L lower leg joint emanation. All lab examination including RA factor was within normal limits, with diminished Serum Vitamin D. HLA B51, ANA were checked and oral mucosal biopsy was done. The most punctual sign exhibited was oral disintegration, various shallow ulcer and scarcely any dissolved knobs in the scrotum. At that point the patient gave joint pain and numbness on right leg. On neurological assessment, a strange motor nerve conduction saw with right tibial neuropathy. At first, doubt with syphilis and tarsal tunnel disorder and following 7-8 days of affirmation, analyzed as Behcet's illness dependent on dermatological, rheumatologic and neurological signs. Treatment given was symptomatic and supportive with pain relievers, corticosteroid, antibiotics, IV fluids, PPI, vitamin supplement, laxative and local anaesthetic. Discussion:Without adequate data it's difficult to examine, in light of anomaly and standardized treatment are questionable at present. New information with respect to its immunopathogenesis, genetics will significantly help in the advancement of research center tests, diagnostic criteria and particularly in the decision of the best treatment

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Genetics of Behçet's Disease

Pathology Research International ◽

10.1155/2012/912589 ◽

2012 ◽

Vol 2012 ◽

pp. 1-6 ◽

Cited By ~ 19

Author(s):

Tamer İrfan Kaya

Keyword(s):

Behçet’S Disease ◽

Disease Susceptibility ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Association Studies ◽

Silk Road ◽

Inflammatory Disorder ◽

Genome Wide Association Studies ◽

Genetic Associations ◽

Behcet's Disease

Behçet's disease (BD) is a systemic inflammatory disorder characterized mainly by recurrent oral and genital ulcers and eye involvement. Although the pathogenesis remains poorly understood, a variety of studies have demonstrated that genetic predisposition is a major factor in disease susceptibility. Peculiar geographical distribution of BD along the ancient Silk Road has been regarded as evidence supporting genetic influence. The observed aggregation of BD in families of patients with BD is also supportive for a genetic component in its etiology. HLA-B51 (B510101 subtype) is the most strongly associated genetic marker for BD in countries on the Silk Road. In recent years, several genome-wide association studies and genetic polymorphism studies have also found new genetic associations with BD, which may have a supplementary role in disease susceptibility and/or severity. The author reviewed the HLA and non-HLA genetic association studies.

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Immunopathogenesis of Ocular Behçet’s Disease

Journal of Immunology Research ◽

10.1155/2014/653539 ◽

2014 ◽

Vol 2014 ◽

pp. 1-13 ◽

Cited By ~ 11

Author(s):

Un Chul Park ◽

Tae Wan Kim ◽

Hyeong Gon Yu

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Retinal Vasculitis ◽

Skin Lesions ◽

Unknown Etiology ◽

Ocular Involvement ◽

Inflammatory Disorder ◽

General Hypothesis ◽

Behcet's Disease

Behçet’s disease (BD) is a chronic recurrent systemic inflammatory disorder of unknown etiology characterized by oral and genital ulcerations, skin lesions, and uveitis. The ocular involvement of BD, or Behçet’s uveitis (BU), is characterized by panuveitis or posterior uveitis with occlusive retinal vasculitis and tends to be more recurrent and sight threatening than other endogenous autoimmune uveitides, despite aggressive immunosuppression. Although pathogenesis of BD is unclear, researches have revealed that immunological aberrations may be the cornerstone of BD development. General hypothesis of BD pathogenesis is that inflammatory response is initiated by infectious agents or autoantigens in patients with predisposing genetic factors and perpetuated by both innate and acquired immunity. In addition, a network of immune mediators plays a substantial role in the inflammatory cascade. Recently, we found that the immunopathogenesis of BU is distinct from other autoimmune uveitides regarding intraocular effector cell profiles, maturation markers of dendritic cells, and the cytokine/chemokine environment. In addition, accumulating evidence indicates the involvement of Th17 cells in BD and BU. Recent studies on genetics and biologics therapies in refractory BU also support the immunological association with the pathogenesis of BU. In this review, we provide an overview of novel findings regarding the immunopathogenesis of BU.

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Behçet’s disease leaves the silk road

Advances in Dermatology and Allergology ◽

10.5114/pdia.2014.40943 ◽

2014 ◽

Vol 6 ◽

pp. 417-420 ◽

Cited By ~ 1

Author(s):

Anna Woźniacka ◽

Piotr Jurowski ◽

Anastazy Omulecki ◽

Marek Kot ◽

Bożena Dziankowska-Bartkowiak

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Silk Road ◽

Behcet's Disease ◽

The Silk Road

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A Darwinian view of Behçet's disease

Rheumatology and Immunology Research ◽

10.2478/rir-2021-0013 ◽

2021 ◽

Vol 2 (2) ◽

pp. 91-99

Author(s):

Rhodri Smith ◽

Robert J. Moots ◽

Mariam Murad ◽

Graham R. Wallace

Keyword(s):

Behçet’S Disease ◽

Complex Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Clinical Findings ◽

Unknown Etiology ◽

Inflammatory Disorder ◽

Protective Mechanisms ◽

Behcet's Disease ◽

Cns Lesions

Abstract Behçet’s disease (BD) is a multisystem inflammatory disorder of unknown etiology, characterized by oral and genital ulceration, with other complications including eye, skin, joint, and central nervous system (CNS) lesions. Diagnosis is based on clinical findings, which may differ between patients. There is a strong genetic basis for BD; however, only a few genes have been associated with the disease across the geographical spread of BD. In this article, we discuss the history and combination of genes involved in this complex disease in relation to the geographical range and present our view that the disease has developed from a Darwinian perspective, with different gene polymorphisms that affect the same biological pathway. Moreover, these mutations individually are protective mechanisms against the disease relevant to each region, which affected both archaic and modern humans.

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Neuro-Behçet’s Disease – Clinical Features, Diagnosis and Differential Diagnosis

European Neurological Review ◽

10.17925/enr.2018.13.2.93 ◽

2018 ◽

Vol 13 (2) ◽

pp. 93

Author(s):

Selen Ozyurt ◽

Petros Sfikakis ◽

Aksel Siva ◽

Cris S Constantinescu ◽

◽

...

Keyword(s):

Differential Diagnosis ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Case Reports ◽

Illustrative Case ◽

Inflammatory Disorder ◽

Behcet's Disease ◽

Multisystem Involvement ◽

The Central Nervous System

Background:Behçet’s disease is a relatively uncommon, inflammatory disorder with characteristic mucocutaneous lesions and multisystem involvement, of unknown aetiology; presumably a vascular autoinflammatory syndrome that develops under combined environmental and genetic influences. As neuro-Behçet’s disease affects the central nervous system in about 10% of cases and in ways that can mimic other neuroinflammatory conditions, awareness of its manifestations, significance, and management is important for neurologists. In March 2017, a mini-symposium at the 11th Congress of Controversies in Neurology in Athens, Greece, was dedicated to specific aspects of Behçet’s and neuro-Behçet’s disease. These included an introduction to Behçet’s disease, pathogenesis and treatment, an overview of its neurological manifestations (neuro-Behçet’s disease) and the differential diagnosis from other neuroinflammatory conditions. Illustrative case reports were used.Objectives:To provide a brief overview of neuro-Behçet’s disease that is informative for clinical neurological practice and that follows the structure of the 2017 mini-symposium.Data sources:Relevant recent comprehensive reviews of the subject and relevant original articles and case reports were provided by each speaker at the mini-symposium. This article contains some of these sources and some additions where necessary to emphasise specific points. References are also provided for more comprehensive recent reviews.Limitations:The mini-symposium was an opportunity for providing a brief update and overview of neuro-Behçet’s disease and to exchange ideas and experience among neurologists. As such, it was found to be helpful, but also limited in scope. This resultant article refers to comprehensive reviews on the topic but is not in itself a comprehensive systematic review.Conclusions:Neuro-Bechet’s disease comprises largely two forms, parenchymal and a non-parenchymal. These manifestations seldom overlap in the same individual and may reflect different pathogenetic mechanisms. The principles of treatment largely follow the principles of treating Bechet’s disease in general, with the mainstay being corticosteroids for exacerbations and immunosuppressive treatments for prevention of exacerbations. One notable exception is cyclosporine, which is typically avoided in neuro-Bechet’s disease. Anti-tumour necrosis factor biologicals play an increasing role in treatment. Distinguishing neuro-Behçet’s disease from other neuroinflammatory conditions, such as multiple sclerosis, is essential for both management and prognostic reasons.

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Decreasing incidence and severity of Behçet’s disease: a changing trend in epidemiological spectrum possibly associated with oral health

TURKISH JOURNAL OF MEDICAL SCIENCES ◽

10.3906/sag-2003-147 ◽

2020 ◽

Vol 50 (7) ◽

pp. 1587-1590

Author(s):

Gonca MUMCU ◽

Fatma ALİBAZ ÖNER ◽

Tülin ERGUN ◽

Haner DİRESKENELİ

Keyword(s):

Oral Health ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Epidemiological Studies ◽

Inflammatory Disorder ◽

Behcet's Disease ◽

Disease Spectrum ◽

Wide Range ◽

Ancient Silk Road

Behçet’s disease (BD) is a systemic and inflammatory disorder that is mainly present along the ancient Silk Road, from the Mediterranean Sea to East Asia. A wide range of prevalence figures (0.1–420/100,000) have been reported for BD, also among Turkish populations of similar genetic background living in different countries. Recently, a decline in the incidence of BD and a change of the disease spectrum to less-severe manifestations have been reported from Japan and Korea, two genetically homogenous, affluent populations with limited immigration. It was hypothesized that a decline in infectious diseases, especially dental/periodontal infections, associated with the improvement in oral health, could be a part of these changes in the disease expression. Further epidemiological studies in other populations might demonstrate whether there is a worldwide similar trend and may provide a better understanding of the triggering factors for the onset and course of BD.

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Relationship between vitamin D level and Bechcet’s disease activity: A systematic review and meta-analysis

International Journal for Vitamin and Nutrition Research ◽

10.1024/0300-9831/a000542 ◽

2020 ◽

Vol 90 (5-6) ◽

pp. 527-534 ◽

Cited By ~ 1

Author(s):

Alireza Khabbazi ◽

Morteza Ghojazadeh ◽

Sakineh Hajebrahimi ◽

Zeinab Nikniaz

Keyword(s):

Vitamin D ◽

Vitamin D Deficiency ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Meta Analysis ◽

Serum Vitamin ◽

Healthy Controls ◽

Behcet's Disease ◽

Serum Vitamin D

Abstract. Aim: In the present study, the evidence about the association between vitamin D deficiency and Behcet’s disease activity was systematically reviewed and meta-analyzed. Method: We searched the English and Persian databases of Medline (Ovid), CINHAL, Scopus, Proquest, the Cochrane library and SID, IranDoc, Magiran, Iran Medex for articles published up until May 2018 with the keywords were related to serum vitamin D and active and inactive Behcet’s disease in adults. Meta-analysis was done using the CMA software. Results: A total of 138 titles were retrieved and reduced to 80 titles after deletion of duplicates and finally after close assessing of titles and abstracts eight eligible studies including a total of 939 participants were identified for systematic review and meta-analysis. According to the results of the meta-analysis, the pooled effect size of the differences in the serum level of vitamin D in patients with inactive Behçet’s Disease and healthy controls was [OR:−0.05; 95% CI:−2.05, 1.94; p = 0.95]. The serum vitamin D level was significantly lower in active patients compared with healthy controls [OR:1.21; 95%CI: −0.12, 2.31; p = 0.03]. The pooled effect size of the differences in the serum level of vitamin D in active and inactive Behçet’s Disease was [OR:−0.71; 95%CI: −1.41, −0.007; p = 0.04] Conclusion: There is an association between vitamin D deficiency and active Behçet’s Disease. Future studies investigating the association of vitamin D deficiency and Behçet’s Disease needs to involve following information: dietary intake of calcium and vitamin D, measuring of sun exposure, report of drug consumption and physical activity level.

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Behçet’s Disease: A Review

Journal of Dental Research ◽

10.1177/154405910508400302 ◽

2005 ◽

Vol 84 (3) ◽

pp. 209-222 ◽

Cited By ~ 59

Author(s):

L.M. Al-Otaibi ◽

S.R. Porter ◽

T.W.J. Poate

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Infectious Agent ◽

Inflammatory Disorder ◽

Autoimmune Reaction ◽

Behcet's Disease ◽

Worldwide Distribution ◽

Life Threatening ◽

Silk Route

Behçet’s disease (BD) is a multi-system inflammatory disorder dominated clinically by recurrent oral and genital ulceration, uveitis, and erythema nodosum. Behçet’s disease runs a chronic course, with unpredictable exacerbations and remissions whose frequency and severity may diminish with time. Behçet’s disease typically arises in young adults, although childhood-onset BD has also been reported. The disease can affect both genders and has a worldwide distribution, although it is more prevalent in countries of the ancient Silk Route. The cause of BD remains unknown, although an autoimmune reaction triggered by an infectious agent in a genetically predisposed individual has been suggested. The treatment of BD is symptomatic and empirical, but generally specific to the clinical features of each patient. The majority of affected individuals do not have life-threatening disease, although mortality can be associated with vascular-thrombotic and neurological disease.

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